WITHDRAWN: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presented as pulmonary-renal syndrome in a coronavirus disease-2019 (COVID-19) patient

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and anti-glomerular basement membrane (GBM) disease are two separate diseases, while sometimes they can coexist together. The exact mechanisms are not clear, but due to the rapid progression and poor prognosis, prompt and aggressive treatment is usually required. We treated with steroids combined with cyclophosphamide and rituximab an 84-year-old man with ANCA-associated vasculitis and anti-GBM disease who had prior pulmonary fibrosis and a coexisting anterosuperior mediastinal mass. Conventional therapy including steroids, plasmapheresis and cyclophosphamide failed to attenuate the anti-GBM disease, yet he responded to an alternative treatment of rituximab. This case suggests the efficacy of steroids and immunosuppressant for the treatment of a dual-positive case with an anterosuperior mediastinal mass.

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ANCA Status or Clinical Phenotype — What Counts More?

Current Rheumatology Reports ◽

10.1007/s11926-021-01002-0 ◽

2021 ◽

Vol 23 (6) ◽

Author(s):

Martin Windpessl ◽

Erica L. Bettac ◽

Philipp Gauckler ◽

Jae Il Shin ◽

Duvuru Geetha ◽

...

Keyword(s):

Clinical Trials ◽

Granulomatosis With Polyangiitis ◽

Clinical Phenotype ◽

Antineutrophil Cytoplasmic Antibody ◽

Cardiovascular Death ◽

Genetic Associations ◽

Ongoing Debate ◽

The Past ◽

Anca Associated Vasculitis

Abstract Purpose of Review There is ongoing debate concerning the classification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. That is, whether classification should be based on the serotype (proteinase 3 (PR3)- or myeloperoxidase (MPO)-ANCA) or on the clinical phenotype (granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)). To add clarity, this review focused on integration of the most recent literature. Recent Findings Large clinical trials have provided evidence that a serology-based risk assessment for relapses is more predictive than distinction based on the phenotype. Research conducted in the past decade indicated that a serology-based approach more closely resembles the genetic associations, the clinical presentation (i.e., lung involvement), biomarker biology, treatment response, and is also predicting comorbidities (such as cardiovascular death). Summary Our review highlights that a serology-based approach could replace a phenotype-based approach to classify ANCA-associated vasculitides. In future, clinical trials and observational studies will presumably focus on this distinction and, as such, translate into a “personalized medicine.”

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An unusual precipitant of acute heart failure—ANCA-associated vasculitis in a patient with ischaemic cardiomyopathy: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa357 ◽

2020 ◽

Author(s):

Krishna Prasad ◽

Pruthvi C Revaiah ◽

Krishna Santosh Vemuri ◽

Parag Barwad

Keyword(s):

Heart Failure ◽

Renal Failure ◽

Acute Heart Failure ◽

Acute Decompensated Heart Failure ◽

Antineutrophil Cytoplasmic Antibody ◽

Left Ventricular ◽

Alveolar Haemorrhage ◽

Coronary Syndrome ◽

Anca Associated Vasculitis ◽

Renal Vasculitis

Abstract Background Antineutrophil cytoplasmic antibody (ANCA)-associated pulmonary renal vasculitis is an uncommon disease entity. Its presentation as acute heart failure for the first time in a patient with established coronary artery disease (CAD) is even rarer. We present here a case of such an association and an approach to managing this clinical situation. Case summary A 60-year-old male patient presented to the emergency room with recent-onset dyspnoea New York Heart Association Class IV. He was having hypertension, uncontrolled diabetes mellitus, chronic kidney disease (CKD), and CAD. He also underwent a percutaneous coronary intervention to left anterior descending in the past for acute coronary syndrome and had moderate left ventricular dysfunction. He was being managed as a case of acute decompensated heart failure (ADHF) and was mechanically ventilated. Suddenly his ventilator requirement increased and endotracheal aspirate contained blood. The chest radiograph showed bilateral hilar infiltrates. Simultaneously he also had recurrent episodes of ventricular tachycardia (VT) requiring direct current (DC) cardioversion. Blood investigations showed deranged renal function and severe hyperkalaemia, but no evidence of coagulopathy. High-resolution computed tomography chest showed features of diffuse alveolar haemorrhage. Further investigations revealed high titres of c-ANCA and raised inflammatory biomarkers. A diagnosis of ANCA-associated vasculitis presenting as acute on CKD with dyselectrolytaemia (hyperkalaemia) leading to VT was made. Apart from standard management for associated illness, he was treated with plasma exchange, steroids, and cyclophosphamide to which he responded and was later on discharged. Discussion Antineutrophil cytoplasmic antibody-related pulmonary renal vasculitis can lead to rapidly progressing renal failure and may present as ADHF in a patient with existent CAD. The associated VT storm in our patient can be attributed to hyperkalaemia secondary to acute renal failure. A multidisciplinary approach is required for the successful management of such a complex clinical scenario.

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ANCA-Associated Glomerulonephritis and Anti-Phospholipid Syndrome in a Patient with SARS-CoV-2 Infection: Just a Coincidence?

Case Reports in Nephrology and Dialysis ◽

10.1159/000517513 ◽

2021 ◽

pp. 214-220

Author(s):

Federica Maritati ◽

Maria Ilaria Moretti ◽

Valentina Nastasi ◽

Roberta Mazzucchelli ◽

Manrico Morroni ◽

...

Keyword(s):

Rapidly Progressive Glomerulonephritis ◽

Kidney Injury ◽

Antineutrophil Cytoplasmic Antibody ◽

Hemodynamic Instability ◽

Tubular Necrosis ◽

Complex Processes ◽

Anca Associated Vasculitis ◽

High Incidence ◽

New Diagnosis ◽

The Relationship

Many reports have described a high incidence of acute kidney injury (AKI) among patients with COVID-19. Acute tubular necrosis has been reported to be the most common damage in these patients, probably due to hemodynamic instability. However, other complex processes may be involved, related to the cytokine storm and the activation of innate and adaptive immunity. Here, we describe a patient who developed an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with rapidly progressive glomerulonephritis and lung involvement and an antiphospholipid syndrome soon after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. After viral pneumonia was excluded by bronchoalveolar lavage, the patient has been treated with rituximab for amelioration of kidney function and resolution of thrombosis without any adverse event. We conclude that COVID-19 may trigger autoimmune diseases including ANCA-associated vasculitis. Thus, this diagnosis should be taken in consideration in COVID-19 patients, especially when they develop AKI with active urinary sediment. In addition, considering the relationship between these 2 diseases, SARS-CoV-2 infection should be excluded in all patients with a new diagnosis ANCA-associated vasculitis before starting immunosuppressive therapy.

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What are the effects of anticytokine targeted therapies for adults with granulomatosis with polyangiitis (GPA) antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis?

Cochrane Clinical Answers ◽

10.1002/cca.3381 ◽

2020 ◽

Author(s):

Sera Tort ◽

Raouf Hajji

Keyword(s):

Targeted Therapies ◽

Granulomatosis With Polyangiitis ◽

Antineutrophil Cytoplasmic Antibody ◽

Anca Associated Vasculitis

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Granulomatosis with Polyangiitis: Recurrence or Treatment Consequences?

European Journal of Case Reports in Internal Medicine ◽

10.12890/2021_002448 ◽

2021 ◽

Author(s):

Andreia Diegues ◽

Joana Tavares ◽

Diogo Sá ◽

João Oliveira ◽

Diana Fernandes ◽

...

Keyword(s):

Intracranial Hypertension ◽

Clinical Improvement ◽

Granulomatosis With Polyangiitis ◽

Antineutrophil Cytoplasmic Antibody ◽

Lung Nodules ◽

Rare Form ◽

Anca Associated Vasculitis

Granulomatosis with polyangiitis (GPA) is the most common antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We describe the case of a 38-year-old woman with relapsing GPA who presented with intracranial hypertension, followed by the appearance of cavitated lung nodules despite treatment with azathioprine. Clinical improvement and ANCA titre reduction were observed after rituximab treatment. We report a rare form of GPA relapse and highlight the challenge of following-up patients with GPA, in whom can be hard to distinguish relapse from the consequences of long-term immunosuppression.

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